I am accustomed to being a zealous advocate in my normal everyday life. That term takes on a whole new meaning when you are fighting for your sick child. The battles with our doctors continue, and I am proud to be my daughter’s zealous advocate.
We weighed the pros and cons, and we decided to get Getty a G-Tube and also a Nissen procedure. The G-Tube is an easy decision, as Getty will lose swallow function some day and the G-Tube will allow her to eat.
The Nissen is designed to prevent future reflux and potential reflux aspiration pneumonia. The procedure is a bit more controversial, but as Kate and I see it, the cost-benefit analysis leads us to the conclusion the Nissen is the way to go. Not getting the Nissen means there is a risk of future reflux aspiration pneumonia, and the risks of getting the Nissen don’t seem so bad in comparison.
The majority of SMA1 kids need a Nissen at some point and SMA1 kids lose strength with each passing day. So it is best to do these procedures now, as a preventative measure, while Getty has the strength to endure the surgery rather than reacting to some potentially horrible situation later, when she may lack the strength to undergo surgery. We scheduled the procedure for mid-November.
The issue is now how to properly do the procedures. An SMA1 child undergoing a surgical operation must have proper nutrition and must have proper ventilation support post-op.
Our gastro thinks a normal glucose IV should be enough. However, the SMA community tells me Getty needs a PPN feed instead. PPN is essentially an IV with additional nutrients in it, not just sugar water.
Our pulmo thinks it is good enough to have a BiPAP breathing machine on stand-by if it appears Getty needs it post-op. However, the SMA community tells me Kaiser needs to put Getty on BiPAP automatically post-op, whether it appears she needs it or not.
I have been studying these issues for a couple weeks, communicating with the SMA community, and communicating with my doctors. I have been trying to push our doctors into following protocols as provided to me by the SMA community.
We met our Kaiser surgeon this week, and he’s a jerk. We were asking him all kinds of questions about proper surgical protocol, and he was offended that we were challenging his opinions based upon information from the SMA community. We told him the information we are getting from the SMA community is based upon input from the handful of leading SMA physicians in the world. He wrote them off as “academics” who publish impractical papers to further their own careers. He told us he agrees with our gastro that there will be no PPN, and he agrees with pulmo that there will be no BiPAP involved. We were shocked to hear for the first time the idea that there will be no BiPAP involved at all.
The next day, I continued to do all I could to advocate for our girl. I got hold of and spoke extensively with the office of Dr. Kathryn Swoboda, one of the top SMA doctors in the world. Her office assured me she was a practicing physician and not just an “academic.” She also told me when their office performs any surgical procedure on an SMA child, they use a PPN feed and they put all SMA kids on BiPAP post-op. In other words, my doctors, who maybe see a handful of SMA cases in a decade, are not following guidelines established by the top SMA doctors in the world, who deal with SMA kids routinely.
I immediately put in calls to our gastro and pulmo to challenge their practices based upon input from Dr. Swoboda’s office. Thankfully, Dr. Swoboda’s office even said they were willing to talk to our Kaiser doctors directly to convince them to change their practices.
I then wrote a cold email to our surgeon to challenge his practices and to inform him he is mistaken to write off Dr. Swoboda and other top SMA doctors as merely “academic.” He wrote me a quick note back saying he simply agreed to do the surgery, and if I had a problem with how it’s recommended then I should take it up with gastro and pulmo. He will follow their instructions.
After leaving messages for gastro and pulmo, I called our pediatrician for help. I told her the problem, and she offered to help. She got a hold of our pulmo, and that led to a conversation between pulmo and me. Pulmo said there must have been be a miscommunication. He was not recommending no BiPAP at all; instead, he was recommending BiPAP as needed based upon post-op observations. This put my mind at ease, somewhat.
The SMA community has been advising me to get Getty on BiPAP pre-op as well as post-op because when you put a kid on BiPAP for the first time, they struggle with the machine and cry a lot. They just are not used to a machine blowing air into their little face. So, if you try to start BiPAP for the first time post-op, then the patient will waste a lot of energy struggling and crying when they should be devoting all their energy to recovery. Pulmo said there is pretty much no way to start BiPAP pre-op, as I had asked.
I was unable to get a hold of gastro to talk about the PPN feed.
The next day, I spoke to another SMA father*. He told me post-op BiPAP should be mandatory and not discretionary based upon post-op observations. An SMA child inherently needs breathing support post-op so they can recover from surgery without having to struggle to breathe. Even if she looks like she’s breathing fine post-op without BiPAP, she is wasting her energy trying to breathe when she should be using her energy trying to recover.
He also confirmed the need for pre-op BiPAP acclimation, for the reasons stated above. He also said we need to get Getty used to a BiPAP machine at home right now so she will be accustomed to it by the time the surgery is done.
This SMA father also told me we need to do a PPN feed post-op because using straight glucose IV does not give SMA kids enough fuel to properly recover.
When his child went to see Dr. Mary Schroth, one of the top SMA doctors in the world, they walked out of her office with a prescription for an at-home BiPAP machine right away. He said an SMA1 diagnosis alone inherently justifies an at-home BiPAP machine prescription.
The bottom line is … our doctors are doing it wrong and they need to be corrected.
What we want is (1) pre-op home BiPAP acclimation; (2) automatic mandatory post-op BiPAP; and (3) a post-op PPN feed. To make matters worse, it is my understanding that there is no harm in doing it this way. Our doctors just don’t see the need and are not doing it based upon our request alone.
This SMA father also told me he would try to get Dr. Schroth involved in our case. I truly hope Dr. Schroth is willing to help set our Kaiser doctors straight with a couple of conference calls. I am honored that this SMA father is helping us and is willing to try to get one of the leading SMA physicians in the world involved on our behalf.
Not only do we have to fight SMA while working full time, we have to fight our doctors. It’s so sad and exhausting. I have spent so much time and energy on these battles, advocating for our Little Owl. If we didn’t have the SMA community in our corner, we would just quietly accept whatever our doctors told us. I feel sorry for SMA parents for having to grapple with SMA in general, but I especially feel sorry for those SMA parents who never get connected to the SMA community. I can only image all the bad medical advice they follow unknowingly.
*Name withheld for privacy.
Hi…..I don’t know your family, and only found out about Getty when Stacey emailed me about joining the craft fair she is doing for you. While I can’t do the fair, I have donated to your family some money, and given Stacey some items to raffle off. I signed on to get the updates on Getty, and it just makes me so sad, that you have to fight so hard for something like this. Having just had a little one added to our family, I can’t begin to know what you and your wife are going through, plus your friends and families. Please know your family is in our thoughts and prayers, and we pray you keep up the strength as little Getty travels down this road. I think what you are doing here, will probably be the path you find your lives taking after Getty is gone. She won’t have died in vain. You are extremely dedicated, and I know that you will be helping other families in the years to come. Bless you both.
The squeaky wheel gets the grease! I work in the health care industry and have come to understand advocacy a whole lot better than I used to. Keep it up, Mark. And doctors – although usually pretty smart and helpful – don’t always have the right answers. As a patient, or voice for a patient, you have the right to question, to refuse, and to seek other opinions. It is exhausting. But keep pushing the envelope to take care of your dear little owl. Blessings to all of you.
Reading this is DEJA VU for our family. Unfortunately, regular docs who know little about SMA think THEIR way is the only way . . . talk to any SMA family and they all will sadly tell you they had to go through the same fight and it’s WRONG! Why do they not learn from other families? Not all SMA children pass away so why not give them a chance? I’m not sure how much I’ve told you about our Stella but she is considered a WEAK Type I and so many docs wrote her off, questioning why we would do a gtube/nissen with the “remaining weeks we had left with our daughter”. They insisted on sugar water IV nutrition for a week, promising that the next day (and then the next . . . and then the next) would be the day that my child would be get fed and guess what? We took our daughter home WEAKER than when we brought her in because they didn’t feed her properly and she lost more muscle mass. Ugh! We had TWO hospitals that wouldn’t prescribe a bipap/cough assist because they didn’t think it would work on such a young child . . . we had a surgeon who placed her surgi-port tell me as I laid her down in the OR that I could take my bipap machine back to the waiting room because they wouldn’t be needing it, only to get a page that they needed it RIGHT NOW. Or how about the time they insisted on using CPAP machine instead of Bipap, only to learn that I was right about my daughter crashing if they used it since it’s like trying to breathe while sticking your head out a car window while driving down the road?? These are stories that so many of us have had to go through and we hope and pray that they will GET IT so noone else has to go through it.
I am SOOO grateful to read how proactive you are! It’s SO exhausting to work to get your doctors on board but you sound like you have the fight and knowledge behind you so that is good. We see Dr. Schroth 2x year and we owe Stella’s life to her team. While exhausting, they start to learn that you actually know what you are talking about. We don’t have NEAR the issues we used to have because they know we are abreast of the leading research out there because WE are the ones fighting for our child every day . . . she’s a blip in their life but she IS OUR LIFE!
As a sidenote, these same people who wrote off my daughter 3 1/2 years ago would be picking up their jaws if they could see my child driving a powerchair today, merely by using tiny sensors in her hands.
If there is anything you need, don’t hesitate to contact any of us . . . we are like family to you and always will be, even if never meet, merely because we share in the same fight and love your child like our own because of it. We are always here for you in thought and prayer.
Stella Turnbull’s family http://www.caringbridge.org/visit/stellaturnbullturnbull
Thank you for the supporting words! It looks like Dr. Schroth is willing to lend us a hand in this fight!
Mark, Your daughter, Getty, is so fortunate to have you and your wife as her zealous advocates. Children, especially, need us to be their zealous advocates. Thank you both for that. Greg and I were in China Town today and we found a little something that reminded us of you and little Getty. We’ll bring it next weekend!
Getty is so blessed to have you both. This fight is so heart breaking but remember that you are likely opening doors so that the next family that your docters incounter will not have such a battle. It is sad that there are more children going through just what Getty is going through but I know you will get the chance to help out another familyone day, and I am cirten that they will be eternaly greatful to have your hand in their fight jut as others continue to help you with yours. You and Kate are my heroes along with your little girl. You are amazing. The same goes for the SMA community who are truly remarcable in their ability to join together with such dilligence, understanding, and compasion. truly remarcable. Thank you for all of the love you spread and share.
Kate and Mark,
Your latest two posts just made my blood boil! Sometimes, as you are well familiar with, doctors are so arrogant that they forget they are actually treating a human being, a child. You are doing the very best for Getty but you must keep fighting. It is always a fight. I hope the appeal process goes in your favor and I pray you are able to see the SMA experts. We will be keeping you all in our thoughts and prayers.